A Multirefractory Lymphomatoid Granulomatosis Successfully Treated with Gemcitabine as a Single Agent: Case Report and Review of the Literature

Abstract

Lymphomatoid Granulomatosis (LYG) is a rare, EBV-driven disease with angiocentric and angiodestructive pleomorphic lymphocytic infiltrates. Pulmonary involvement is frequent and responsible for pulmonary manifestations which may be associated with systemic symptoms. Its rarity makes it difficult to diagnose, and the diagnosis is usually made after several months of investigation. There is no consensus on treatment, which can range from a waitand- see approach to multidrug therapy with allogeneic hematopoietic stem cell transplantation, according to histological grade. The lack of consensus does not help in the management of these patients. We report the case of a patient with a multirefractory LYG, who achieved a Complete Remission (CR) with gemcitabine as single agent salvage treatment.